Exploring the Role of Carbonic Anhydrase IX as Diagnostic and Theranostic Target in Von-hippel Lindau Disease
The study is a phase 2, non-comparative and non-randomized, single arm, national clinical trial testing the hypothesis that CAIX-PET has diagnostic and theranostic potential in VHL disease and in VHL-/- tumors. Participants will receive a single dose of the diagnostic radiopharmaceutical \[89Zr\]Zr-DFO-Girentuximab and subsequently will be subjected to imaging with an hybrid PET/CT scanner. Sensitivity and diagnostic accuracy of experimental imaging will be assessed against standard of truth derived from standard of care procedures such as MRI or pathology following surgery. A theranostic approach will be simulated by replacing the physical decay of the diagnostic isotope \[89Zr\]Zr with the physical decay of therapeutic isotopes and evaluating tissue dosimetry.
• Voluntarily given informed consent
• Age ≥18 years old
• Performance Status ECOG/WHO score 0-2
• For females of reproductive potential, negative pregnancy test and use of highly effective contraception for 30 days following IMP administration
• For males of reproductive potential, use of highly effective contraception for 30 days following IMP administration.
⁃ And, for the primary cohort:
• Diagnosis of VHL disease requiring surveillance following confirmation of pathogenic variant at genetic test
⁃ Alternatively, for the secondary cohort:
⁃ \- Clinical and/or pathological diagnosis of hemangioblastoma, pheochromocytoma, pancreatic neuroendocrine tumor or clear cell renal cell carcinoma requiring surgery.